Evolution of cerebrovascular imaging and associated clinical findings in children with Alagille syndrome.

PURPOSE: Alagille syndrome (ALGS) is a multisystem autosomal dominant disorder with highly variable expression. Intracranial arterial and venous anomalies have a reported prevalence of 30-40% and can increase the risk of stroke by 16%. Few reports document the frequency and evolution of cerebrovascular abnormalities (CVAs) in children with ALGS. We aimed to define the spectrum, frequency, and evolution of CVAs in a series of children with ALGS using magnetic resonance angiography (MRA). METHODS: We conducted a single-center, retrospective study in a large tertiary pediatric hospital. CVAs were grouped into 4 categories: 1) Stenosis or narrowing; 2) Aneurysms and ectasias; 3) Tortuosity; and 4) Vascular anomalies and anatomical variants. RESULTS: Thirty-two children met the inclusion criteria. The median age at initial diagnosis was 6 (3.8-10.3) years. Thirteen (40%) had follow-up MRI at a mean of 55 (31.5-66) months. Eighteen (56%) had CVAs; the most frequent fell into group 1 (n = 12, 37.5%). CVAs were stable over time, except for one patient with Moyamoya arteriopathy (MMA). One patient developed a transient ischemic attack secondary to an embolic event. Three (9.3%) had microhemorrhages at the initial diagnosis secondary to Tetralogy of Fallot. Another patient had recurrent subdural hematomas of unknown cause. CONCLUSION: CVAs were stable except in the presence of MMA. Vascular strokes, which are reported in older patients with ALGS, were not a common feature in children under 16 years of age, either at presentation or over the 31.5-66 month follow-up period.

Expanding the Imaging Spectrum of Polymorphous Low-Grade Neuroepithelial Tumor of the Young in Children.

Polymorphous low-grade neuroepithelial tumors of the young (PLNTY) are rare brain tumors first described in 2017 and recently included in the 2021 5th World Health Organization Classification of Tumors of the Central Nervous System. They typically affect children and young adults. Few pediatric cases have been reported in the literature. The most common imaging features described, include location within the temporal lobe, involvement of the cortical/subcortical region, coarse calcifications, and well-defined margins with solid and cystic morphology, with slight-or-no enhancement. However, there is limited information on imaging features in children. We present the imaging spectrum of neuroimaging features in a series of pediatric patients with a histologically and molecularly proved PLNTY diagnosis. Coarse calcifications are uncommon in children compared with the adult literature, and they may develop with time. The transmantle-like sign can be observed, and adjacent cortical dysplasia may be seen. Seizure recurrence may occur despite gross total resection of the tumor.

T2-based magnetic resonance genitography in children with complex anorectal malformations: experience at a tertiary pediatric hospital in Latin America.

In children with anorectal malformations (ARMs), it is essential to have a diagnostic imaging method that helps with the evaluation of the internal anatomy. In patients with a persistent cloaca, an ARM variant, in which the measurement of the urethral channel and common channel determines surgical management, there are multiple options for imaging. Magnetic resonance imaging (MRI) is an excellent method for this purpose, from which accurate measurements of channel length can be obtained. Additionally, the use of volumetric/isotropic sequences allows multiplanar reformatting. We present our experience with pelvic MRI and intracavitary non-paramagnetic contrast (MR genitography). This method uses volumetric T2-weighted images and the instillation of saline solution as a contrast agent to distinguish the common channel, length of the urethra, anatomy of the vagina, and presence and location of the rectal fistula. We believe this technique to be particularly useful for those working in settings with limited MRI resources.

Computed tomography angiography features of children with ectopia cordis.

Ectopia cordis is a rare congenital defect with high mortality, and it remains challenging to radiologists, neonatologists and surgeons. CT angiography provides key information that aids in the decision-making process for possible surgical intervention. This pictorial essay describes CT angiography features in six neonates with ectopia cordis.

Is horseshoe lung a component of VACTERL spectrum? Case report and review of literature.

Horseshoe lung (HL) is a rare congenital anomaly that has been classically associated with Scimitar syndrome. Very few cases have been described in the context of the VACTERL spectrum. We present a case of a newborn girl with mesocardia, tracheoesophageal fistula, and imperforated anus, who required O2 support at birth and during hospitalization. A chest CT angiography revealed a HL as an incidental finding. We suspect that HL and the VACTERL spectrum, are not separated entities but likely a further expansion of VACTERL-associated symptoms. HL might be underdiagnosed in asymptomatic patients as Chest CT angiography is not part of the routine work up for patients with VACTERL association.

[Burkitt's lymphoma of the skull calotte: a case report]. / Linfoma de Burkitt de la calota craneana: a propósito de un caso.

Introduction: Burkitt's lymphoma is a non- Hodgkin B-cell lymphoma whose cranial location is extremely rare. Clinical case: The case of a 35-year-old Peruvian man with a progressive parietal left cranial tumor is described. A biopsy with immunohistochemistry was performed for the diagnostic confirmation of Burkitt's lymphoma. First-line outpatient treatment with CODOX-M/IVAC was initiated, however, he stopped attending his treatment for 2 months. He returns, he is shown progression of the disease and is treated with rituximab, ifosfamide carboplatin and etoposide. Does not respond satisfactorily and dies. Conclusions: Burkitt's lymphoma is a very aggressive tumor that, if not treated in a timely manner, has high mortality, as happened with the patient in the case.

Introducción: El linfoma de Burkitt es un linfoma no Hodgkin de células B cuya ubicación craneal es extremadamente rara. Caso clínico: Se describe el caso de un hombre peruano de 35 años con una tumoración craneal parietal izquierda de crecimiento progresivo. Para la confirmación diagnóstica de linfoma de Burkitt se realizó una biopsia con inmunohistoquímica. Se inició tratamiento ambulatorio de primera línea con CODOX-M/IVAC, sin embargo, deja de acudir a su tratamiento por 2 meses. Retorna, se le evidencia progresión de la enfermedad y se le trata con rituximab, ifosfamida carboplatino y etopósido. No responde de forma satisfactoria y fallece. Conclusiones: El Linfoma de Burkitt es un tumor muy agresivo que si no es tratado oportunamente presenta alta mortalidad, tal como ocurrió con el paciente del caso.

[Tomographic characteristics of solid-pseudopapillary neoplasms (Frantz tumor).] / Características tomográficas del tumor sólido pseudopapilar de páncreas (tumor de Frantz).

Introduction: Solid-pseudopapillary neoplasms (SPN), or Frantz tumor, is a rare, low-grade neoplasm that occurs mainly in young women. It was described in 1959 by Virginia Frantz and constitutes 0.2-2.7 % of all pancreatic tumors. Computed tomography (CT) plays an important role in the diagnosis of this pathology of scarce reporting. The objective of the present study is to describe the epidemiological and tomographic characteristics of the SPN in the Instituto Nacional de Enfermedades Neoplásicas (INEN) of Peru. Methods: Descriptive cross-sectional study performed with medical records of all patients diagnosed with SPN between 2004 and 2014. The variables described were tumor size, location, shape, borders, thickness of the capsule, composition, calcifications and uptake of contrast. The categorical variables were expressed in absolute and relative frequencies; while the numerical variables were described with median and interquartile deviation (ID). Statistical support STATA Version 12.0 was used. Results: Of all pancreatic cystic tumors (PCT), 51.9% corresponded to confirmed cases of TSP. The median age was 23.5 years. The isolated location in the head predominated (33.3%); the most frequent mixed location was body and tail (16.7%); diameter was more frequent between 5.1-10 cm and the content of the majority was predominantly solid. 30.0% of the NSP presented calcifications. Conclusion: Most cases of INP SPN (2004-2014) have similar characteristics to those reported in the international literature.

Introducción: El tumor sólido pseudopilar de páncreas (TSP), o tumor de Frantz, es una neoplasia rara de bajo grado que acontece principalmente en mujeres jóvenes. Fue descrita en 1959 por Virginia Frantz y constituye del 0.2-2.7% de todos los tumores pancreáticos. La tomografía computada (TC) desempeña un papel importante en el diagnóstico de esta patología de escaso reporte. El objetivo del presente estudio es describir las características epidemiológicas y tomográficas del TSP en el Instituto Nacional de Enfermedades Neoplásicas (INEN) del Perú. Métodos: Estudio transversal descriptivo realizado con historias clínicas (HC) de todos los pacientes con diagnóstico de TSP entre los años 2004 y 2014. Las variables descritas fueron tamaño del tumor, ubicación, forma, bordes, grosor de la cápsula, composición, calcificaciones y captación de contraste. Las variables categóricas fueron expresadas en frecuencias absolutas y relativas; mientras que las variables numéricas fueron descritas con mediana y desviación intercuartílica (DIC). Se utilizó como soporte estadístico STATA Versión 12.0. Resultados: De todos los tumores quísticos de páncreas (TQP), el 51.9 % correspondió a casos confirmados de TSP. La mediana de la edad fue 23.5 años. Predominó la ubicación aislada en cabeza (33.3 %); la ubicación mixta más frecuente fue la de cuerpo y cola (16.7 %); fue más frecuente el diámetro entre 5.1-10 cm y el contenido de la mayoría fue a predominio sólido. El 30.0 % de los TSP presentaron calcificaciones. Conclusión: La mayoría casos de TSP del INEN (2004-2014) tienen características similares a lo reportado en la literatura internacional.

Descripción de la conducta sexual en adultos jóvenes limeños / Description of sexual behaviors in Peruvian younger adults

Objetivo: Describir las conductas sexuales en una muestra de adultos jóvenes limeños de nivel socioeconómico C y D. Diseño: Estudio descriptivo transversal. Lugar: EIGER. Participantes: 156 adultos jóvenes. Intervenciones: Se usó un cuestionario semi-estructurado auto-administrado y se realizó el análisis con el Software STATA 9.0. Principales medidas de resultados: Para comparar las variables cuantitativas de acuerdo a variables cualitativas dicotómicas se usó el test T de Student, previo examen de los supuestos de normalidad por el test de Shapiro Wilk y de homogeneidad de varianzas por el test de Levene, de no cumplirse los supuestos se usó el test de suma de Rangos de Wilxocon. Para evaluar la asociación entre dos variables cualitativas se usó el Test exacto de Fisher. Resultados: La edad promedio de inicio sexual fue de 17.23 ±3.51 años. La primera relación sexual se da con la pareja sentimental, el número promedio de parejas sexuales 1.88 ±1.01 en mujeres y 4.35 ±3.57 en hombres. Tener más de una pareja sexual durante la misma época, usar el Internet para buscar parejas sexuales, acudir a los servicios de prostitución y tener RS con parejas ocasionales conocidas o desconocidas son conductas mayoritariamente practicadas por hombres en nuestro estudio. Conclusiones: La población masculina se encuentra en mayor riesgo por presentar conductas y actitudes más riesgosas que la población femenina.

Objective: To describe sexual behavior patterns in a sample of Peruvian young adults of socioeconomic levels C and D. Study Design: Descriptive, cross- sectional study. Participants: 156 young adults Interventions: We used a self-completed questionnaire and analyzed it with the statistical software package STATA. Main outcome measures: The T test of Student was used to compare the quantitative variables according to the dichotomy qualitative variables, with a previous examination of the suppositions of normality by the test of Shapiro Wilk and the homogeneity of variances by the test of Levene. If the suppositions were not completed, we used the test of sum of Ranges of Wilxocon. To evaluate the association between two qualitative variables, the exact Test of Fisher was used. Results: Respondents have on average, started their sexual life by the age of 17.23 ±3.51 years. Mostly, the initial intercourse is with their partner ; the average number of sexual partners is 1.88 ±1.01 in women and 4.35 ±3.57 in men. To have more than one sexual partner at the same time, to use the Internet with a sexual purpose, to attend prostitution services and to have intercourse with occasional well-known or strangers are behavior patterns practiced mainly by men in our study. Conclusions: Male population is more threatened because of high-risk behavior and attitudes than female population.